Brainstem glioma

Last revised by Dr Mohamed Saber on 19 Feb 2021

Brainstem gliomas consist of a heterogeneous group which vary greatly in histology and prognosis.

Unless otherwise specified the term brainstem glioma usually refers to the most common histology, the diffuse brainstem glioma, and in children this is most likely a diffuse midline glioma H3 K27M–mutant

Brainstem gliomas account for ~25% of all posterior fossa tumors and are most common in children between 7 and 9 years of age 5. There is no recognized gender or racial predilection.

Brainstem gliomas are also recognized in adults, although they are rare accounting for only 2% of adult brain tumors 7. They typically occur in younger adults (third and fourth decade) and tend to be of low grade (WHO I or II) 7.

Although the exact presentation will vary according to location and size of the tumor, in general patients will exhibit a combination of  4:

The duration of symptoms is usually much shorter in diffuse gliomas, in which the history is typically very short (a few days) 4. Additionally, diffuse gliomas more frequently have multiple cranial nerve palsies.

Recognized histological types include:

The most frequently used classification system is to divide these tumors into four types 5:

  1. diffuse brainstem glioma
  2. focal brainstem glioma
  3. (dorsally) exophytic
  4. cervicomedullary
    • probably an artificial group made up of the downward extension of true brainstem gliomas or upward extension of upper cervical cord intramedullary spinal cord tumors 5

As a general rule mesencephalic tumors tend to be of a lower grade than those in the pons and medulla 3.

  • pontine
  • mesencephalic
  • medullary
    • least common location
    • includes focal dorsally exophytic, focal, diffuse and cervicomedullary junction variants
    • cervicomedullary junction tumors usually represent upper cervical tumors extending superiorly
    • most common location for NF1 associated tumors

MRI is the imaging modality of choice. The appearance will vary with the tumor type, thus please refer to individual articles. 

May show anterior displacement of the basilar artery.

Again, both treatment and prognosis are significantly influenced by tumor type, morphology and location. Radiation is a key part of treatment.

As a general rule, dorsal exophytic tumors and cervicomedullary tumors tend to do best with surgery, whereas surgery has no role in the management of diffuse brainstem gliomas

  • diffuse
    • terrible prognosis
    • 90-100% patients die within 2 years of diagnosis 6
  • focal (tectal glioma)
    • excellent long-term survival with CSF shunting (essentially benign lesions)
  • focal (other)
    • good long-term prognosis with surgery
  • (dorsally) exophytic tumors
    • good long-term prognosis with surgery​

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Cases and figures

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